Material and methods: All patients with open heart surgery under CPB was enrolled in a case-control study. RD is defined as creatinine clearance <60 ml/min. 8 risk factors such as pre-operative RD, duration of CPB and of aortic clamping, core temperature, hematocrit, perfusion pressure, hemoglobinuria, use of vasopressors. Results: 5 independent risk factors of RD during CPB are perfusion pressure below 50 mmHg lasting over 30 min (adjusted OR 8.77), CPB time> 120 min (adjusted OR 6.35), aortic clamping >60 min (adjusted OR 4.16), pre-operative RD (adjusted OR 2.98), hemoglobinuria (adjusted OR 2.68). Conclusions: 5 independent risk factos of RD during CPB are long and low perfusion pressure, long duration of CPB and of aortic clamping, pre-operative RD and hemoglobinuria.
Thoracic Surgery, Fanconi Syndrome, Risk Factors

Adenine ; analogs & derivatives ; Fanconi Syndrome ; Humans ; Organophosphonates

Adefovir dipivoxil (ADV) is a nucleotide used as long-term therapy of chronic hepatitis B. Many published reports have shown that long-term high-dose therapy with adefovir can be associated with proximal renal tubular dysfunction resulting in significant hypophosphatemia, renal insufficiency and osteomalacia. We have encountered two patients who developed evidence of hypophosphatemic osteomalacia while on long-term low-dose adefovir therapy for chronic hepatitis B. We report on its clinical features and its potential resolution with cessation of the drug and supplementation with phosphate. We also reviewed the other published cases associated with hypophosphatemic osteomalacia after low-dose adefovir therapy. The symptoms and the hypophosphatemia improved after cessation of the drug and supplementation with phosphate in most cases. Patients taking adefovir long-term should receive regular investigation of the phosphate level and renal function.
Fanconi Syndrome ; Hepatitis B, Chronic* ; Hepatitis, Chronic* ; Humans ; Hypophosphatemia ; Kidney Diseases ; Osteomalacia* ; Renal Insufficiency

Crystalline nephropathy is a rare yet well-known condition associated with multiple myeloma and other light chain–secreting disorders. Paraproteins that are resistant to proteolysis crystallize within proximal tubular cells and cause light-chain proximal tubulopathy, which presents clinically as Fanconi syndrome. Podocytes are rarely affected, and the crystalline inclusions within podocytes are typically precipitated, yielding significant glomerular proteinuria. Here we report a case of extensive crystalline inclusions primarily within podocytes and proximal tubules that presented only with Fanconi syndrome and renal insufficiency. Despite the presence of extensive crystalline inclusions in podocytes and diffuse foot process effacement, the patient had no clinical evidence suggestive of podocyte injury.
Crystallins* ; Fanconi Syndrome ; Foot ; Humans ; Multiple Myeloma* ; Paraproteins ; Podocytes ; Proteinuria* ; Proteolysis ; Renal Insufficiency

Fanconi-Bickel syndrome (FBS, OMIM 227810), a rare autosomal recessive disorder of carbohydrate metabolism, is caused by SLC2A2 (GLUT2) mutations. The study reported 3 cases of FBS who were confirmly diagnosed by SLC2A2 gene analysis. The three patients showed typical features like glycogen storage disease and proximal renal tubular nephropathy. Homozygous splice-site mutation IVS8+5G>C (c.1068+5 G>C) was found in patient A and homozygous nonsense mutation c.1194T>A (p.Tyr398X) in patient B. Patient C harboured a missense mutation c.380C>A (p.Ala127Asp) and a de novo insertion c.970dupT (p.324TyrfsX392) which was not inherited from her parents. Four mutations were identified in the 3 Chinese FBS patients. Except IVS8+5G>C mutation, the other 3 mutations were novel in Chinese population. To the best of our knowledge, patient C may be the first FBS case worldwide with de novo mutation.
Fanconi Syndrome ; genetics ; Female ; Glucose Transporter Type 2 ; genetics ; Humans ; Mutation

Acute Kidney Injury ; etiology ; Fanconi Syndrome ; etiology ; Female ; Humans ; Lymphoma, Non-Hodgkin ; complications ; Middle Aged

Ifosfamide-induced nephrotoxicity is usually manifested in the form of Fanconi syndrome and the combination of cisplatin enhances ifosfamide-induced nephrotoxicity. We here report a case of report specific proximal tubular dysfunction and progressive renal failure after ifosfamide and cisplatin chemotherapy in a 32-year old woman with ovarian cancer. The patient was referred to our department due to severe hypokalemia and elevated serum creatinine. Her renal function was abruptly impaired and serum and urine electrolytes were consistent with Fanconi syndrome. The kidney biopsy revealed atrophy and falling of renal tubular cells, and immunohistochemical staining with aquaporin 1 (AQP1) revealed that injured epithelial cells were proximal tubular cells. This finding suggests that ifosfamide selectively impairs proximal tubule function and combination with cisplatin causes progressive renal failure
Aquaporin 1 ; Atrophy ; Biopsy ; Cisplatin ; Creatinine ; Electrolytes ; Epithelial Cells ; Fanconi Syndrome ; Female ; Humans ; Hypokalemia ; Ifosfamide ; Kidney ; Ovarian Neoplasms ; Renal Insufficiency

Cadmium (Cd)-induced renal damage primarily affects the cellular and functional integrity of the proximal tubules. Cd intoxication is a rare cause of Fanconi syndrome. We report a 31-year-old woman with Fanconi syndrome confirmed by laboratory findings and a renal biopsy that also showed an elevated urinary Cd. Seven months before admission, the patient had a normal urinalysis and renal function. Six and 3 months before admission, the patient ingested Chinese herbal mixtures. On admission, the blood Cd concentration was normal, and the urine Cd concentration was 58 microgram/g of creatinine, 29-times higher than the normal range. A renal biopsy showed degeneration of the proximal tubules with normal glomeruli. The serum creatinine had increased from 1.2 to 3.2 mg/dL over 8 months. This case of Fanconi syndrome and rapidly progressive renal damage over a short period was associated with elevated urinary Cd levels.
Adult ; Asian Continental Ancestry Group ; Biopsy ; Cadmium ; Creatinine ; Fanconi Syndrome ; Female ; Humans ; Reference Values ; Renal Insufficiency ; Urinalysis

Cystinosis, an autosomal recessively inherited lysosomal storage disease, results from impaired transport of the amino acid cystine out of cellular lysosomes. The consequent accumulation and crystallization of cystine destroys tissues, causing growth retardation, Fanconi syndrome, renal failure, eye problems, and endocrinopathies. The gene for cystinosis, CTNS, was mapped to chromosome 17p13. The diagnosis of cystinosis was made by measuring the leukocyte cystine content. The presence of typical corneal crystals on slit-lamp examination is also diagnostic. Since treatment with cysteamine has proved extremely effective, early diagnosis and treatment are critical aspects. We experienced a typical case of cystinosis in a 12-year-old boy with growth retardation.
Child ; Crystallization ; Cysteamine ; Cystine ; Cystinosis* ; Diagnosis ; Early Diagnosis ; Fanconi Syndrome ; Humans ; Leukocytes ; Lysosomal Storage Diseases ; Lysosomes ; Male

Tenofovir disoproxil fumarate (TDF) is one of the most widely used treatment options for human immunodeficiency virus (HIV) and HBV infections. Despite its efficacy and safety, some cases of nephrotoxicity have been reported in the treatment of HIV patients. Even more recently, very few cases of Fanconi syndrome associated with tenofovir therapy in HBV monoinfection have been reported. Herein, we report a case of a 47-year-old male with an HBV monoinfection, who developed Fanconi syndrome and a secondary osteomalacia with multiple bone pain. After TDF withdrawal and supplementation of calcitriol, his renal function was reverted. Although the overall risk of TDF-associated nephrotoxicity is very low, both glomerular and tubular function should be monitored in patients undergoing TDF treatment.
Calcitriol ; Fanconi Syndrome* ; Hepatitis B virus ; Hepatitis B, Chronic* ; Hepatitis, Chronic* ; HIV ; Humans ; Kidney Tubules ; Male ; Middle Aged ; Osteomalacia ; Tenofovir